Antihemophilic Factor / Von Willebrand Factor Dosage

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Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Hemophilia A

Minor hemorrhage (early joint or muscle bleed or severe epistaxis): Loading dose 15 IU FVIII/kg to achieve FVIII plasma level of approximately 30% of normal; one infusion may be sufficient. If needed, half of the loading dose may be given once or twice daily for 1 to 2 days.

Moderate hemorrhage (advanced joint or muscle bleed, neck, tongue or pharyngeal hematoma (without airway compromise), tooth extraction, or severe abdominal pain): Loading dose 25 IU FVIII/kg to achieve FVIII plasma level of approximately 50% of normal, followed by 15 IU FVIII/kg every 8 to 12 hours for first 1 to 2 days to maintain FVIII plasma level at 30% of normal, and then the same dose once or twice a day for a total of up to 7 days, or until adequate wound healing.

Life-threatening hemorrhage (major operations, gastrointestinal bleeding, neck, tongue or pharyngeal hematoma with potential for airway compromise, intracranial, intraabdominal or intrathoracic bleeding, or fractures): Initially 40 to 50 IU FVIII/kg, followed by 20 to 25 IU FVIII/kg every 8 hours to maintain FVIII plasma level at 80 to 100% of normal for 7 days, then continue the same dose once or twice a day for another 7 days in order to maintain the FVIII level at 30 to 50% of normal.

Usual Pediatric Dose for von Willebrand's Disease

Treatment of von Willebrand Disease:
Type 1 mild, if desmopressin is inappropriate (Baseline von Willebrand factor (VWF) activity typically greater than 30%):
Major Hemorrhage (e.g. severe or refractory epistaxis, GI bleeding, CNS trauma, or traumatic hemorrhage): Loading dose 40 to 60 IU/kg, then 40 to 50 IU/kg every 8 to 12 hours for 3 days to keep the trough level of VWF greater than 50%; then 40 to 50 IU/kg daily for a total of up to 7 days of treatment.
Type 1 moderate or severe (Baseline VWF activity typically less than 30%):
Minor (e.g. epistaxis, oral bleeding, menorrhagia): 40 to 50 IU/kg (1 or 2 doses)
Major (e.g. severe or refractory epistaxis, GI bleeding, CNS trauma, hemarthrosis or traumatic hemorrhage): Loading dose 50 to 75 IU/kg, then 40 to 60 IU/kg every 8 to 12 hours for 3 days to keep the trough level of VWF greater than 50%; then 40 to 60 IU/kg daily for a total of up to 7 days of treatment. Factor VIII levels should be monitored and maintained according to the guidelines for hemophilia A therapy.
Types 2 (all variants) and 3:
Minor (clinical indications above): 40 to 50 IU/kg (1 or 2 doses).
Major (clinical indications above): Loading dose of 60 to 80 IU/kg, then 40 to 60 IU/kg every 8 to 12 hours for 3 days to keep the trough level of VWF greater than 50%; then 40 to 60 IU/kg daily for a total of up to 7 days of treatment. Factor VIII levels should be monitored and maintained according to the guidelines for hemophilia A therapy.

In the case of emergency surgery, administer a loading dose of 50 to 60 IU/kg and, subsequently, closely monitor the trough coagulation factor levels.

Prevention of Excessive Bleeding During and After Surgery in VWD:
When possible, it is recommended that the incremental in vivo recovery (IVR) be measured and that baseline plasma VWF and FVIII be assessed in all patients prior to surgery. Measure IVR as follows:
1. Measure baseline plasma VWF.
2. Infuse 60 IU VWF/kg product intravenously at time 0.
3. At time +30 minutes, measure plasma VWF.
IVR = (Plasma VWF at time +30 min - Plasma VWF at baseline) / 60 IU kg.

Calculation of the loading dose requires four values: the target peak plasma VWF level, the baseline VWF level, body weight (BW) in kilograms, and IVR. When individual recovery values are not available, a standardized loading dose can be used based on an assumed VWF IVR of 2.0 IU/dL per IU/kg of VWF product administered.

Major Surgery: VWF Target Peak Plasma Level is 100 IU/dL and FVIII Target Peak Plasma Level is 80 to 100 IU/dL.
Calculation of Loading Dose (to be administered 1 to 2 hours before surgery): (Target peak plasma VWF - baseline plasma VWF x BW (kg) / IVR = IU VWF required.
If the incremental IVR is not available, assume an IVR of 2 IU/dL per IU/kg and calculate the loading dose as follows: (100 - baseline plasma VWF) x BW (kg) / 2.
Minor surgery VWF Target Peak Plasma Level is 50 to 60 IU/dL and FVIII Target Peak Plasma Level is 40 to 50 IU/dL.
Calculation of Loading Dose (to be administered 1 to 2 hours before surgery): (Target peak plasma VWF - baseline plasma VWF x BW (kg) / IVR = IU VWF required.

Subsequent Maintenance Doses for the Prevention of Excessive Bleeding During and After Surgery:
Major surgery: VWF target level is greater than 50 IU/dL up to 3 days following surgery and greater than 30 IU/dL after day 3. FVIII target trough plasma level is greater than 50 IU/dL up to 3 days following surgery and greater than 30 IU/dL after day three. The minimum duration of treatment is 72 hours.
Minor surgery: VWF target level is greater than or equal to 30 IU/dL up to 3 days following surgery. FVIII target trough plasma level is greater 30 IU/dL after day three. The minimum duration of treatment is 48 hours.
Oral Surgery: VWF target level is greater than 30 IU/dL up to 3 days following surgery. FVIII target trough plasma level is greater than or equal to 30 IU/dL after day three. The minimum duration of treatment is 8 to 12 hours.

Usual Pediatric Dose for Hemophilia A

Adequate and well-controlled studies with long-term evaluation of joint damage have not been done in pediatric subjects. Joint damage may result from suboptimal treatment of hemarthroses. For immediate control of bleeding for Hemophilia A, the general recommendations for dosing and administration for adults, as follows, may be referenced.

Minor hemorrhage (early joint or muscle bleed or severe epistaxis): Loading dose 15 IU FVIII/kg to achieve FVIII plasma level of approximately 30% of normal; one infusion may be sufficient. If needed, half of the loading dose may be given once or twice daily for 1 to 2 days.

Moderate hemorrhage (advanced joint or muscle bleed, neck, tongue or pharyngeal hematoma (without airway compromise), tooth extraction, or severe abdominal pain): Loading dose 25 IU FVIII/kg to achieve FVIII plasma level of approximately 50% of normal, followed by 15 IU FVIII/kg every 8 to 12 hours for first 1 to 2 days to maintain FVIII plasma level at 30% of normal, and then the same dose once or twice a day for a total of up to 7 days, or until adequate wound healing.

Life-threatening hemorrhage (major operations, gastrointestinal bleeding, neck, tongue or pharyngeal hematoma with potential for airway compromise, intracranial, intraabdominal or intrathoracic bleeding, or fractures): Initially 40 to 50 IU FVIII/kg, followed by 20 to 25 IU FVIII/kg every 8 hours to maintain FVIII plasma level at 80 to 100% of normal for 7 days, then continue the same dose once or twice a day for another 7 days in order to maintain the FVIII level at 30 to 50% of normal.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dialysis

Data not available

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